Disseminated Intravascular Coagulation
The 5 Minute Pediatric Consult
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Disseminated Intravascular Coagulation |
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Sadhna M. Shankar
DEFINITION
Disseminated intravascular coagulation (DIC) is a profound hemorrhagic disorder associated with concurrent activation of procoagulant, anticoagulant, and fibrinolytic mechanisms.
PATHOPHYSIOLOGY/PATHOLOGY
Dysregulation of procoagulant, anticoagulant, and fibrinolytic pathways due to various initiating events.
- Microvascular hemorrhages and thrombosis.
- Procoagulant factors released in the blood may initiate DIC in acute promyelocytic leukemia.
- Endotoxins released during infections cause activation of coagulation factor XII, endothelial injury, platelet aggregation, and inhibition of fibrinolysis.
- Following disease processes can cause DIC:
- Infections
- Meningococcemia
- Gram negative and positive sepsis
- Malaria
- Viral and fungal sepsis
- Tissue injury
- Massive brain injury
- Shock or asphyxia
- Hypothermia or hyperthermia
- Malignancies
- Acute promyelocytic leukemia
- Metastatic carcinomas
- Obstetric causes
- Intrauterine fetal death
- Amniotic fluid embolism
- Gastrointestinal
- Fulminant hepatitis
- Reyes syndrome
- Neonatal
- Necrotizing enterocolitis
- Congenital viral infections
- Group B strep infection
- Erythroblastosis fetalis
- Miscellaneous
- Acute hemolytic transfusion reaction
- Kasabach Merritt syndrome
- Snake bite
EPIDEMIOLOGY
- Exact incidence not known.
- Most commonly secondary to infections.
- Snake bite may be most common cause world wide.
COMPLICATIONS
- Hemorrhage
- Renal failure
- Multiorgan system failure
PROGNOSIS
Poor unless underlying disease is treated.
- Coagulopathy of liver disease
- Pathological fibrinolysis
- Thrombotic thrombocytopenic purpura
HISTORY
- Presence of one of the underlying conditions listed in causes.
- Abrupt onset of bleeding?
- Pulmonary or intracranial hemorrhage?
- Oliguria
- Prolonged bleeding from venipuncture sites.
- Major organ dysfunction
- Pulmonary, renal, hepatic.
- Signs of underlying disease.
- Generally a very sick child.
- Ecchymosis and petechiae.
- Bleeding from previously intact venipuncture sites.
- Skin infarctions (purpura fulminans) secondary to thrombosis of dermal vessels.
- Pulmonary hemorrhage, gastrointestinal bleeding, bleeding from surgical wounds, hematuria.
- Intraperitoneal and pleural hemorrhages.
All these tests should be checked every 8 to 12 hours as they change rapidly.
Test: CBC
Significance: Decreased platelet count, often earliest abnormality.
Test: Peripheral smear
Significance: Schistocytes, microspherocytes (50% of cases).
Test: PT, PTT, and Thrombin time
Significance: Prolonged
Test: Fibrinogen
Significance: Decreased
Test: Fibrin degradation products
Significance: Increased
Test: Factor VIII and V
Significance: Decreased, Factor VIII is normal in coagulopathy of liver disease.
- Treat the underlying disorder.
- Cryoprecipitate, platelets and fresh frozen plasma to control bleeding.
- Fresh frozen plasma also replaces anticoagulantsprotein C and S.
- Heparin used for management of purpura fulminans due to meningococcemia.
- Routine use of heparin for DIC is debatable.
SUPPORTIVE CARE
Manage other organ system failure.
ICD-9-CM 286.6
Levi M, van der Poll T, van Deventer SJ. Pathogenesis of disseminated intravascular coagulation in sepsis. JAMA 1993;270(8):975979.
Wheeler A, Rubenstein EB. Current management of disseminated intravascular coagulation. Oncology 1993;8(9):6979.
Wintrobes clinical hematology, 10th ed., Vol. II. Baltimore: Lippincott Williams & Wilkins, 1998, 14801493.
Copyright © 2000 Lippincott Williams & Wilkins
M. William Schwartz, Louis M. Bell, Jr., Peter M. Bingham, Esther K. Chung, David F. Friedman and Andrew E. Mulberg, The 5 Minute Pediatric Consult